Multiple guidelines have been published over the last few years for the diagnosis and management of cystic fibrosis (CF) and cystic fibrosis related diabetes (CFRD), although some of the recommendations are based on extrapolation from other forms of diabetes and/or expert opinions. This document seeks to combine the guidelines to provide an Australian approach to the management of CFRD and establish the guidelines within the Australian CF Standards of Care. It is intended that this document will provide assistance to doctors, nurses, dietitians, physiotherapists, diabetes educators and CF patients concerning the issues surrounding CFRD, and will be reviewed and updated in 2016.

Changes in the plasma concentration of C-reactive protein were assessed as a diagnostic test for sepsis in critically ill patients. Forty-nine episodes of secondary sepsis were identified in 31 patients. In 43 out of the 49 episodes there was a 25% or greater change in the concentration of C-reactive protein on the day that sepsis was diagnosed but in six episodes of sepsis the change was less than 25%. A 25% rise in the plasma concentration of C-reactive protein in the absence of other non-infective causes of a raised C-reactive protein, such as inflammation, tissue injury or surgery, is highly suggestive of infection, but failure of the C-reactive protein to rise does not eliminate a diagnosis of sepsis.

Aim:  To document current practice in the nutritional management of cystic fibrosis in Australia and New Zealand and to examine changes in practice since 1996. Methods:  Thirty‐four cystic fibrosis services in Australia and New Zealand responded to a survey that examined current nutritional management practices and dietetic staffing levels. The questionnaire was based on a previous survey conducted in 1996. Results:  Cystic fibrosis dietetic staffing levels were low. No service met the staffing level recommended by the UK Cystic Fibrosis Trust. Minor practice variations existed in nutrition assessment and monitoring: nutrition support and vitamin supplementation. Changes in the management of pancreatic enzyme replacement therapy and cystic fibrosis‐related diabetes were reported since 1996. Conclusion:  This survey highlights that improvements in consistency of nutrition management have been achieved since 1996 and reflect adherence to available clinical guidelines for pancreatic enzyme replacement therapy. While there are many areas of agreement in nutrition care for people with cystic fibrosis around Australia and New Zealand, there are still practice differences, implying that Australian‐ and New Zealand‐specific clinical guidelines are warranted. The implementation of the Australasian Clinical Practice Guidelines for Nutrition in Cystic Fibrosis will be important in standardising and improving cystic fibrosis care.

Relatively high ambient temperature may play a role in the pathogenesis of DIOS and constipation in CF.

Background Adults with cystic fibrosis (CF) have been reported to be at five to ten-fold risk (25 to 30 fold risk after solid organ transplant) of colorectal cancer (CRC) than the general population. Limited publications to date have reported on practical aspects of achieving adequate colonic cleanse producing good visualisation. In this study, we compared two bowel preparation regimens, standard bowel preparation and a modified CF bowel preparation. Methods A non-randomised study of adults with CF attending a single centre, requiring colonoscopy investigation were selected. Between 2001 and 2015, 485 adults with CF attended the clinic; 70 adults with CF had an initial colonoscopy procedure. After five exclusions, standard bowel preparation was prescribed for 27 patients, and modified CF bowel preparation for 38 patients. Demographic and clinical data were collected for all consenting patients. Results There was a significant difference between modified CF bowel preparation group and standard bowel preparation group in bowel visualisation outcomes, with the modified CF bowel preparation group having a higher proportion of “excellent/good” GI visualisation cleanse (50.0% versus 25.9%) and lower rates of “poor” visualisation cleanse (10.5% versus 44.5%) than standard bowel preparation ( p = 0.006). Rates of “fair” GI cleanse visualisation were similar between the two groups (39.4% versus 29.6%) (Additional file 1 : Table S1). Detection rates of adenomatous polyps at initial colonoscopy was higher in modified CF bowel preparation cohort than with standard preparation group (50.0% versus 18.5%, p < 0.01). Positive adenomatous polyp detection rate in patient’s age > 40 years of age was higher (62.5%) than those < 40 years of age (24.3%) ( p = 0.003). Colonic adenocarcinoma diagnosis was similar in both groups. Conclusion This study primarily highlights that standard colonoscopy bowel preparation is often inadequate in patients with CF, and that colonic lavage using modified CF bowel preparation is required to obtain good colonic visualisation. A higher rate of polyps in patients over 40 years of age (versus less than 40 years) was evident. These results support adults with CF considered for colonoscopy screening at 40 years of age, or prior to this if symptomatic; which is earlier than CRC screening in the non-CF Australian population. Electronic supplementary material The online version of this article (10.1186/s12876-019-0979-z) contains supplementary material, which is available to authorized users.